本文报道了6例主一肺动脉间隔缺损,其中1例经尸体解剖证实,其余5例均由手术证实。全部病例均经心血管造影检查并由此作出明确诊断。本文通过这6例病例的回顾性分析,总结出此疾病在心血管造影术中的八种表现,并对各种表现的形成机制进行了讨论。将此疾病分为三型,对于I型及Ⅲ型,首选轻度右前斜位或正位升主动脉造影,对于Ⅱ型则首选侧位升主动脉造影。 This article reports 6 cases of primary pulmonary artery defect, of which 1 case confirmed by autopsy, and the remaining 5 cases were confirmed by surgery. All cases were examined by cardiovascular angiography and thus make a clear diagnosis. This retrospective analysis of these 6 cases summarizes the eight manifestations of this disease in cardiovascular angiography and discusses the mechanisms of various manifestations. The disease is divided into three types, for type I and type III, the preferred mild right anterior oblique or ascending aortography, for type II is the preferred lateral ascending aortography.